Primary intraosseous carcinoma of the head and neck is rare, and lesions isolated to the anterior maxilla are obviously even more rare. The diagnosis of intraosseous carcinoma can be difficult, and it must be based on strict criteria. We report the case of a 40-year-old man who was referred for definitive treatment of a cancerous lesion of the anterior maxilla. The patient's mass had initially been thought to be a benign odontogenic cyst. The patient was treated with surgery and prosthetic rehabilitation. We describe the clinical presentation, pertinent workup including imaging, and the treatment of this case, and we review the literature.